neurofibromatosis is a benign neurogenic tumor, originatig from schwann cells of the nerve sheath. this tumor forms a round, white mass on the course of the involved nerve. it occurs sporadically or in association with von recklinghausen's disease. laryngeal involvement is a rare occasion that affects women more than men (f/m ratio 3:2). the most common symptoms are hoarseness, dyspnea and dysp...

A 52-year-old male with a past medical history of hypertension presented with a two-week history of mild right lower quadrant abdominal pain. He denied chronic nonsteroid anti-infl ammatory drug use, alcohol or tobacco abuse. No skin lesions or any areas of hypo/hyperpigmentation were noted. Abdominal exam revealed mild tenderness in the right lower quadrant. Laboratory data including serum sod...

BACKGROUND Neurofibromatosis type 1 (NF1) is an autosomal-dominant inherited disease, characterised by the development of nerve sheath tumors. NF1 is the most frequently inherited disease associated with a predisposition for cancer (in particular malignant peripheral nerve sheath tumors: MPNST). NF1 is a progressive disease with phase-like growth spurts of dermal or plexiform neurofibroma (PNF)...

Segmental neurofibromatosis (SNF) is a rare type of neurofibromatosis (NF-1) resulting from post-zygotic somatic mutations in the neurofibromin gene that leads to mosaicism. Reported manifestations of SNF include neurofibromas, freckling, or café-au-lait spots limited to a single body region or limb. We present a 5-month-old male referred to our clinic for evaluation of congenital excessive ski...

Stem cells are under strict regulation by both intrinsic factors and the microenvironment. There is increasing evidence that many cancers initiate through acquisition of genetic mutations (loss of intrinsic control) in stem cells or their progenitors, followed by alterations of the surrounding microenvironment (loss of extrinsic control). In neurofibromatosis type 1 (NF1), deregulation of Ras s...

Neurofibromatosis type 1 (NF-1), also known as von Recklinghausen disease is caused by disorder of a single gene on chromosome 17 that usually restrains cell division. The NF1 gene is mapped to chromosome 17(17p21). Its protein product neurofibromin, negatively regulate oncogenic Ras, and thus regulate cell proliferation. Neurofibromas are heterogeneous and complex benign tumours, consisting of...